title: Hemophagocytic lymphohistiocytosis in total parenteral nutrition dependent children: description of 5 cases and practical tips for management.
abstract: Although total parenteral nutrition (TPN) is mandatory in children with intestinal failure, this treatment is not risk free. The main complications of TPN include catheter-related sepsis, thrombosis, hepatic cholestasis and cirrhosis, metabolic bone disease, and, rarely, reactive hemophagocytic lymphohistiocytosis (HLH). The pathogenesis of HLH in patients with TPN is not known, although some authors hypothesized that it can result from the activation of macrophages because of "fat overload." We reported 5 cases of HLH that occurred in patients with 4 different underlying disorders, all requiring TPN for a long term. In our series, an underlying immunological defect or a serious infection (sepsis) can have triggered HLH. Therefore, it could be reasonable to hypothesize that besides TPN in itself, minor immune defects and infections may act together by overcoming a threshold of immune stimulation, which ultimately leads to HLH.
authors.affiliation: *Department of Pediatrics, University of Trieste †Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Pastore
authors.forename: Francesca
authors.initials: F
authors.lastname: Barbieri
authors.forename: Grazia
authors.initials: G
authors.lastname: Di Leo
authors.forename: Erica
authors.initials: E
authors.lastname: Valencic
authors.forename: Alberto
authors.initials: A
authors.lastname: Tommasini
authors.forename: Alessandro
authors.initials: A
authors.lastname: Ventura
mesh_terms: D002675:Child, Preschool; D005227:Fatty Acids / Q000008:administration & dosage / Q000009:adverse effects*; D005260:Female; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000276:immunology / Q000628:therapy*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy* / Q000209:etiology* / Q000276:immunology; D008297:Male; D010289:Parenteral Nutrition, Total / Q000009:adverse effects*; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D005227:Fatty Acids; D013256:Steroids